Dual endothelin receptor antagonist bosentan, which is known to be safe and effective in adults with pulmonary hypertension (PH), has also been shown to help pediatric PH patients, according to a study published in the British medical journal Heart.
In the retrospective, observational study, researchers treated 40 children with bosentan. Twenty had idiopathic pulmonary arterial hypertension (IPAH) and 20 had PH associated with other conditions, such as congenital heart disease, parenchymal lung or connective tissue disease, or HIV. Their mean age was 8.3 years.Twenty-five of the children were administered bosentan as first line treatment, and nine were given intravenous epoprostenol.
In the IPAH group, 19 children were stabilized with bosentan treatment alone, but 12 patients required combined treatment with epoprostenol. Children with secondary PH showed the most improvement, and bosentan was generally well-tolerated by all the children, noted the study.